نتايج بلند مدت آدرنالكتومي تام براي بيماري كوشينگ

Long Term Results of Bilateral Adrenalectomy for Cushingʹs Disease

Introduction: Various investigations of total (bilateral) adrenalectomy for Cushingʹs disease have yielded different results. Previous studies showed a different incidence and prognosis of Nelsonʹs syndrome. The aim of this study is to investigate and present the long term results of total adrenalectomy for bilateral adrenocortical hyperplasia (Cushingʹs disease). Materials and Methods: During a ten-year period between 1991-2001, a total of 54 patients with Cushingʹs syndrome were diagnosed and operated upon at teaching hospitals of Tabriz University of Medical Sciences and followed at endocrine clinics. Thirty-nine patients (27 females and 12 males) with Cushingʹs disease and a mean age of 32. 6 years, underwent total adrenalectomy and were followed for one to ten years (mean duration of follow up 6. 4 years). Results: The remission rate following bilateral adrenalectomy was 100%. In 2 patients after a period of steroid replacement therapy, there were signs of steroid overdosage. Replacement therapy was discontinued in both. Classic Nelsonʹs syndrome characterized by severe and progressive mucocutaneous hyperpigmentation, very high levels of plasma ACTH and invasive macroadenoma as diagnosed by pituitary CT or MRI scan developed in 2 patients (5%) 4 and 6 years after adrenalectomy. In another 2 patients (5%), despite severe hyperpigmentation and very high levels of serum ACTH, there was no pituitary mass at imaging (non-classical Nelsonʹs syndrome). Thus a total of 4 patients (10.2%) with a mean age of 23 years developed Nelsonʹs syndrome during the follow up period. All patients were females. Three patients (two females and one male) died of colonic carcinoma, pancreatic malignancy, and metastatic adenocarcinoma of unknown origin with intraperitoneal seeding and liver metastasis. Conclusion: We conclude that total adrenalectomy is an effective method of correction of hypercortisolism in Cushingʹs disease. The incidence rate of Nelsonʹs syndrome following total adrenalectomy in the present study is lower than that reported in previous studies. It seems that younger females are at a higher risk of developing Nelsonʹs syndrome.